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Abstract: SA-PO871

Glomerulonephritis (GN) Due to Systemic Lupus Erythematosus in a Seronegative Patient: A Lupus Criteria Dilemma

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Wajid, Sumbal, Albany Medical Center, Albany, New York, United States
  • Grigoryan, Lilit, Albany Medical Center, Albany, New York, United States
  • Pal, Aman, Albany Medical Center, Albany, New York, United States
  • Peredo-Wende, Ruben A., Albany Stratton VA Medical Center Albany, Albany, New York, United States
Introduction

Systemic Lupus Erythematosus (SLE) is a well-recognized autoimmune disease with varying clinical and immunological manifestations. According to the 2019 American College of Rheumatology lupus consensus criteria, a positive ANA at any time is required as an entry criterion. Our case highlights a clinical and pathological diagnosis of SLE with negative ANA and anti-dsDNA.

Case Description

A 54-year-old African-American woman presented with cough, dyspnea, orthopnea, and leg swelling. Initial work up showed hemoglobin 10.2 g/dL, unremarkable EKG, troponin, and BNP. CT chest showed a large pericardial effusion, with TTE confirming tamponade physiology. A pericardial window was performed. All infectious work up including blood and pericardial fliuid cultures remained negative. Further testing was pursued that showed positive direct Coombs test, proteinuria and hematuira on urine analysis, and low C3 and C4 levels. This prompted further work up for renal disease in the context of multisystem disease. Serology came back negative for ANA, anti-dsDNA, ENA panel, and antiphospholipid antibodies, as well as c-ANCA, p-ANCA, anti-MPO and anti-PR3 antibodies. Protein electrophoresis and hepatitis panel were unremarkable. Ultimately, kidney biopsy revealed diffuse mesangial and focal segmental endocapillary proliferative GN with positive immunofluorescence to IgG, IgM, C3 and C1, suggesting immune-complex type GN. Although serologic work up remained negative, a presumptive diagnosis of SLE with lupus nephritis was made, in setting of Coombs positive anemia, serositis, hypocomplementemia and immune-complex type GN. Patient was started on steroids and mycophenolate mofetil. 7 years later, she remains on mycophenolate mofetil with stable creatinine and kidney function.

Discussion

The 2019 EULAR/ACR classification criteria diagnose SLE with a score of 10 or greater. However, these criteria exclude SLE if the ANA is negative. If we were to follow this updated criteria for the diagnosis of SLE, our patient would not be classified as having lupus, even though their score is well over 20, neglecting the negative ANA. Although ANA-negative lupus is quite rare, with only a prevalence of around 2%, it is still crucial to properly diagnose these patients as both early diagnosis and initiation of therapy can greatly benefit our patients.